product summary
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company name :
MyBioSource
product type :
antibody
product name :
FAA Antibody
catalog :
MBS853069
quantity :
0.1 mg
price :
355 USD
clonality :
monoclonal
host :
mouse
conjugate :
nonconjugated
reactivity :
human
application :
western blot, ELISA, enzyme immunoassay
more info or order :
MyBioSource product webpage
product information
catalog number :
MBS853069
products type :
Antibody
products full name :
FAA Antibody
products short name :
FAA
other names :
fumarylacetoacetase; Fumarylacetoacetase; fumarylacetoacetase; FAA; beta-diketonase; fumarylacetoacetate hydrolase (fumarylacetoacetase); Beta-diketonase; Fumarylacetoacetate hydrolase
products gene name :
FAA
other gene names :
FAH; FAH; FAA
uniprot entry name :
FAAA_HUMAN
clonality :
Monoclonal
isotype :
IgG2a
host :
Mouse
sequence length :
419
specificity :
Anti- FAA recognizes FAA expression in HEK293 whole cell lysate.
form :
Each vial contains 100 ug IgG in 100 ul of citrate-tris-HCl buffer pH7.0 with 0.02% Proclin 300.
storage stability :
The antibody may be stored at -20 degree C for one year in its original formulation. Additionally, antibody may be stored at 2 degree C to 8 degree C for up to 1 month without detectable loss of activity. Avoid repeated freeze-thaw cycles of the diluted antibody.
tested application :
ELISA (EIA),Western Blot (WB-Ce)
other info1 :
Source Note: Purified mouse monoclonal antibody generated using E Coli expressed recombinant protein FAA as immunogen.
other info2 :
Immunogen: Recombinant protein corresponding to full length FAA.
products categories :
Plant antibody
ncbi gi num :
4557587
ncbi acc num :
NP_000128.1
ncbi gb acc num :
NM_000137.2
uniprot acc num :
P16930
ncbi mol weight :
38,614 Da
ncbi pathways :
Metabolic Pathways (132956); Metabolism Pathway (477135); Metabolism Of Amino Acids And Derivatives Pathway (106169); Phenylalanine And Tyrosine Catabolism Pathway (106189); Tyrosine Degradation, Tyrosine = Homogentisate Pathway (522534); Tyrosine Degradation, Tyrosine = Homogentisate Pathway (468237); Tyrosine Metabolism Pathway (82959); Tyrosine Metabolism Pathway (325); Tyrosine Degradation I Pathway (142386); Tyrosine Degradation I Pathway (139217)
ncbi summary :
This gene encodes the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia (HT). [provided by RefSeq, Jul 2008]
uniprot summary :
FAH: Defects in FAH are the cause of tyrosinemia type 1 (TYRO1). An inborn error of metabolism characterized by elevations of tyrosine in the blood and urine, and hepatorenal manifestations. Typical features include hepatic necrosis, renal tubular injury, episodic weakness, self-mutilation, and seizures. Renal tubular dysfunction is associated with phosphate loss and hypophosphataemic rickets. Progressive liver disease can lead to the development of hepatocellular carcinoma. Dietary treatment with restriction of tyrosine and phenylalanine alleviates the rickets, but liver transplantation has so far been the only definite treatment. Belongs to the FAH family. Protein type: Amino Acid Metabolism - tyrosine; EC 3.7.1.2; Hydrolase. Chromosomal Location of Human Ortholog: 15q25.1. Cellular Component: cytosol. Molecular Function: fumarylacetoacetase activity; metal ion binding. Biological Process: L-phenylalanine catabolic process; tyrosine catabolic process; arginine catabolic process. Disease: Tyrosinemia, Type I
size1 :
0.1 mg
price1 :
355 USD
more info or order :
MyBioSource product webpage
company information
MyBioSource
P.O. Box 153308
San Diego, CA 92195-3308
sales@mybiosource.com
https://www.mybiosource.com
1-888-627-0165
headquarters: USA
MyBioSource, LLC was orginally founded in Vancouver by three enthusiastic scientists who are passionate about providing the world with the best reagents available. Together, they form a company with a big vision known as MyBioSource. MyBioSource is now located in San Diego, California, USA.

"MyBioSource's number 1 vision is to be the world's number 1 quality reagents provider."

Our goal is to provide researchers, scientists and customers alike with a one-stop-shop for all of their reagents needs, whether it is monoclonal antibody, polyclonal antibody, recombinant protein, peptide, etc...

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